In October 2011, KJT Group was commissioned by the IPPF to conduct an awareness and diagnostic pathways survey. Diagnosed pemphigus and pemphigoid (P/P) patients (N=87) completed a 15-minute online survey. Of patients surveyed, 25.3% were male and 74.7% were female. The average age of patients surveyed was 55 years, with 67.8% having pemphigus and 32.2% having pemphigoid. Roughly half (54%) of patients were diagnosed within the last 12 months and 46% were diagnosed within the last 12-36 months. All patients were from the United States.
- Almost 80% of patients sought medical attention within the first three months of symptom onset.
- The majority (63.2%) of patients’ initial symptoms were lesions in and/or on the mouth, lip, gums, or throat.
- Dentists were one of the top clinicians seen first, with 23% of patients seeing a dentist as their first clinician. Almost half of patients (46%) saw a dentist at some point regarding their symptoms.
- On average, patients saw five healthcare providers in pursuit of a correct diagnosis, and 10% reported seeing more than 10 healthcare providers.
- On average, it took patients 10 months to achieve a correct diagnosis.
- More than half of patients reported extreme difficulty in finding a doctor knowledgeable enough to accurately diagnose (56%) and effectively treat (51.2%) their condition.
- Of patients who saw a dentist, almost half (46%) reported they were not knowledgeable about their P/P symptoms and performed no action relevant to their P/P. Forty-percent reported being referred to another healthcare provider, and 13% said they received a diagnosis.
- The majority (83%) of patients reported having a negative experience when seeking a diagnosis.
- More than half (60.1%) of patients felt the time it took them to be diagnosed was too long.
- Almost all patients (96.6%) were unaware of P/P prior to their diagnosis.
- When asked to indicate the one emotion that best described their experience from the time symptoms appeared to receiving an accurate diagnosis, 49.4% of patients said “frustrated”; 18.4% said “anxious”; and 11.5% said “confused.” Only 1.1% said “satisfied.”
Another study,* published in December 2000 by Dr. David Sirois, et al., surveyed 99 PV patients and found that 80% of patients experienced oral lesions as the first sign of PV, and 24% of patients only experienced oral lesions. Oral PV was less commonly recognized than cutaneous PV, with diagnostic delays commonly being greater than six months in duration.
*Sirois, D. et al., 2000. Archives of Dermatology. Vol 136. “Diagnostic Patterns and Delays in Pemphigus Vulgaris: Experience with 99 Patients.”